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Neurodevelopmental Outcome in High-Risk Congenital Diaphragmatic Hernia Patients: An Appeal for International
Kitty G Snoek1, Irma Capolupo, Annabella Braguglia
1Intensive Care and Department of Pediatric Surgery, Erasmus MC-Sophia Children's Hospital, Rotterdam, The Netherlands.
Insights
Most children with congenital diaphragmatic hernia (CDH) achieve normal cognition by age two, but motor function delays are common. Standardized follow-up is crucial for identifying risk factors and reducing long-term morbidity in CDH survivors.
Area of Science:
- Pediatric Surgery
- Neonatal Medicine
- Developmental Pediatrics
Background:
- Mortality rates for congenital diaphragmatic hernia (CDH) are declining.
- Focus is shifting towards improving neurodevelopmental outcomes in CDH survivors.
- Understanding long-term morbidity is essential for high-risk CDH patients.
Purpose of the Study:
- To evaluate the neurodevelopmental outcomes of high-risk CDH patients.
- Assessed outcomes in patients treated under the CDH EURO Consortium standardized protocol.
- Investigated cognitive and motor function at 12 and 24 months.
Main Methods:
- Observational, prospective cohort study in two European centers.
- 88 CDH patients assessed using Bayley Scales of Infant Development (BSID).
- Neurodevelopment evaluated at 12 and 24 months; center-specific data analyzed separately.
Main Results:
- Most patients exhibited normal cognitive function by 24 months (70.7%-97.4%).
- Motor function was delayed in a significant proportion of patients (45.7%-89.8% at 24 months).
- Longer hospital stay, low socioeconomic status, and ethnicity were associated with poorer cognitive outcomes.
Conclusions:
- CDH patients typically have normal cognition but are at risk for motor delays by age two.
- Variability in outcomes between centers necessitates cautious interpretation.
- Multicenter collaboration for standardized follow-up is recommended to reduce morbidity.
Background:
Since mortality in congenital diaphragmatic hernia (CDH) is decreasing, morbidity such as neurodevelopmental outcome is becoming increasingly important.
Objectives:
We evaluated neurodevelopmental outcome in high-risk CDH patients treated according to the CDH EURO Consortium standardized treatment protocol.
Methods:
This observational, prospective cohort study was conducted in two European centers. Neurodevelopment of 88 patients (Rotterdam n = 49; Rome n = 39) was assessed at 12 and 24 months with the Bayley Scales of Infant Development (BSID)-II-NL (Rotterdam) or BSID-III (Rome). Data of the centers were analyzed separately.
Results:
Cognition was normal in 77.8% of children from Rotterdam and in 94.8% from Rome at 12 months, and in 70.7 and 97.4%, respectively, at 24 months. Motor function was normal in 64.3% from Rotterdam and in 81.6% from Rome at 12 months and in 45.7 and 89.8%, respectively, at 24 months. Longer length of hospital stay (LoS) was associated with worse cognitive outcome and motor function; LoS, low socioeconomic status, and ethnicity were associated with lower cognition.
Conclusions:
At 2 years, most CDH patients have normal cognition, but are at risk for motor function delay. Due to differences in outcomes between centers, careful interpretation is needed before conclusions can be drawn for other centers. Future multicenter collaboration should not only focus on standardization of postnatal care, but also on international standardization of follow-up to identify risk factors and thereby reduce morbidity.

